Monday, December 28, 2015

Never a dull moment around here. In the midst of our round of home IV antibiotics, Grandma Carol, Grandpa Dudley and Uncle Jared arrived from New York for Christmas. Grandma Carol and Grandpa Dudley had been planning to come for Christmas for a while, and we'd almost cancelled their visit when we were admitted to the hospital. After we got discharged from the hospital, though, we figured we should just stick to the original plan. Uncle Jared had some unused vacation time to burn before the year's end, and apparently felt like a full immersion in our chaotic household was just the thing to soothe his nerves at the end of the year. All in all it worked out surprisingly well, given that we were running home IV's at the same time as all these people were in and out of our house. I'm especially glad that the grandparents made it out, since it was the first time they met Lime, and they hadn't seen Lemon in quite some time. Grandma Carol got right into the swing of things, watching videos with Lemon while he did his various treatments.

On Wednesday, we went to the clinic for a follow up visit. Since Lemon seemed to be back at baseline, we crossed our fingers and had the PICC pulled. It was an uncomfortable procedure and Lemon handled it amazingly well. Now we're just keeping up with his normal maintenance routine and waiting to see if the IV antibiotics did the trick. We've also added Flonase to his daily schedule in the hopes that it will help keep his nose from running and break the runny nose - cough - antibiotics cycle that we have been on too many times. On Thursday, we celebrated Christmas a day early, before Grandma and Grandpa went back to New York. So many toys, so little time.

And, that more or less brings us to today, where somewhat against our better judgement but pulled along by the lure of friends we haven't seen in entirely too long, we drove off into the teeth of Winter Storm Goliath to fly back to Boston. The fact that I am posting this from a hotel room in our old neighborhood means that we made it. Don't ask me how. I will say that traveling with the vest was difficult in a way that none of us had anticipated. TSA and the airline didn't make so much as a peep about it. Leave it to the two year old to find a way to make life more interesting. The compressor for the vest packs into a rolling case about the same size and design as a regular carry-on, and while we were waiting at the gate before boarding, Lemon decided to stick his head between the two upright parts of the handle on the case and was temporarily stuck, much to his great dismay. Hopefully the experience was traumatic enough that he won't make the same mistake again!

It is definitely weird to be walking around these old familiar streets after a year's absence. Our hotel is across the parking lot from our old pediatrician's office, where we received Lemon's CF diagnosis. Right after we arrived this afternoon, I ran across the street to CVS to get whole milk and high-calorie snacks, and had a vivid flashback of the hour we spent in the very same store, two and a half years ago, fighting with our insurance company to get Lemon's first prescription for enzymes filled. It's an odd lens through which we CF mamas view the world, that's for sure. Now I'm enjoying a coffee mug of white wine from our old local wine store (keepin' it classy!) and hoping that once the kids adapt to their new surroundings, their behavior will return to normal so that the friends we've traveled so far to visit won't deem us unfit parents.

I note, without a shred of regret, that this is the last post for 2015. It's been quite a year, and I won't be sorry to have this one in the rear view. Let's see what 2016 has to offer--I wish all of you the very best of health and happiness in the year to come.

Monday, December 21, 2015

It's been a week of a few more surprises, not the least of which is that I'm writing this post from home, at my own computer.

Surprise 1: I thought I knew what tired was. I was wrong.
Surprise 2: I thought we had a complicated medication schedule before. I was wrong.
Surprise 3: I thought I knew just how resilient and generally amazing Lemon is. I was wrong.

On Tuesday of last week, we had a PICC placed. After the rodeo-type scenario that was the IV placement, we decided to put the PICC in under general anesthesia, rather than just sedation. Since Lemon was going to be down in interventional radiology anyhow, we took chest and abdominal X-rays, too. It cracked me up that the radiologist, who was younger than I, referred to it as a "chest film" even though I'm quite sure he's never touched a physical piece of film during his time as a physician. In any event, the PICC placement went very smoothly and the X-rays looked normal, so that was a win overall. On Wednesday, the doctors evaluated Lemon and his home team, and decided that we could be discharged to finish up the IV's at home. Hooray! We were discharged Thursday afternoon. By the end of the day Thursday, we'd received a giant bag of supplies from the home health pharmacy, and a visit from a nurse to teach us how to use them.
Overall I am delighted to be home. It is vastly better for the kids--they can live their normal lives with a minimum of disruption. For Papa Bear and me, this existence really stretches the definition of normal, but I'm more than willing to stretch if it keeps my kids feeling like regular kids. Here's what happens in our house these days (in order to keep this to a manageable length, I'll just focus on what happens between 5:30-6:30 a.m., and you can just use your imagination to fill in the 3 additional meals, 2 additional IV treatments at 8 hour increments, and 2 additional vest/nebulizer treatments that happen at other times of day, plus the usual shenanigans of caring for a 5-month-old infant and keeping a household running).

5:30. Get up. Wash hands and put on gloves. Connect tubing to new syringe of antibiotic, load syringe and tubing into pump. Prime two saline syringes and one heparin syringe. Lay out other assorted IV supplies on tray. Assemble two neb cups and masks. Load with hypertonic saline and Pulmozyme.

5:45. Go into Lemon's room. Get therapy cart out of closet, plug in power strip and compressor for vest. Slip vest onto Lemon by opening one shoulder so as not to interfere with the tubes coming out of his right arm. Sterilize connector on the PICC with an alcohol prep pad. Flush line with one of the saline syringes. Connect tube from antibiotic syringe to the connector. Connect hoses to vest. Administer two puffs of albuterol. Start vest. Connect neb cup with hypertonic saline to compressor and start compressor. Check to make sure antibiotic is really running.

6:10. Switch to Pulmozyme neb cup. Express enthusiasm for the remarkable rendition of "Wheels on the Bus" that we are watching on YouTube.

6:20. Power down vest and disconnect hoses. Put on fresh gloves. Disconnect tubing from the PICC. Connect saline syringe to the PICC and administer the first flush, disconnect and then connect heparin syringe for the second flush. Remove vest. Gather up disposable debris and put in trash. Remove gloves. Take off vest. Stow cart and various supplies in places where a two year old will not destroy them.

6:30. Breakfast. Prepare Scandishake. Mix applesauce, probiotics, and enzymes. Convince Lemon to ingest the aforementioned. Load syringes with ranitidine and Zyrtec. Convince Lemon to ingest the aforementioned. Locate multivitamin and vitamin D solutions. Convince Lemon to ingest the aforementioned. Locate coffee cup. Discover that coffee is cold. Put in microwave for 33 seconds. Ahhh. Ready for the day to begin.
The good news is that Lemon does seem to be more or less back to baseline. We have a follow-up appointment at the clinic on Wednesday, and my guess is that they will take the PICC out. Then, we'll watch and wait. Hopefully all this has been worth it, and whatever has been causing all Lemon's troubles will be gone, and we can return to our version of normal. Although I wouldn't recommend this lifestyle as an overall fitness plan, it has had one noticeable side effect--two kids later, I could fit into my wedding dress today (if I still owned it, that is).

Monday, December 14, 2015

For a brief moment last week it looked like we might have kicked this bug. After adding hypertonic saline to our daily routine, Lemon was able to get back to his baseline. Unfortunately, once the oral antibiotic ended, the runny nose returned with the cough hard on its heels. I knew pretty much as soon as his nose started to run that we were going to end up being admitted, but we managed to hang on in a state of semi-denial until Sunday morning. Then, knowing that an admission was inevitable, we decided to just head in rather than waiting at home for Lemon's symptoms to get any worse. I think that was the right call, especially since Sunday afternoon is a pretty quiet time at the hospital and we were able to get ourselves and all our stuff situated without too much trouble.

It required the combined effort of 6 adults to get Lemon's IV placed, but after that trauma it hasn't seemed to bother him much. He is receiving Zosyn, a broad-spectrum antibiotic, which hopefully should kill whatever is causing his troubles. We're also doing 4 vest treatments per day in an effort to shake out whatever we can and help the antibiotics work.

There are a lot of things that are unsurprising about this hospitalization so far: terrible food, constant interruptions by various staff members, blood sugar monitoring at 2am, etc.

Some things have surprised me, though. One is the amount of attention it requires to keep a two-year-old from pulling out his IV by accident. Lemon has been amazingly good about not messing with it on purpose, but he moves around a lot and those little tubes seem to want to get twisted and snagged on every possible point of entanglement. And then there's the pole and the wire that plugs it in and the furniture and the toys and the other people in the room. Another thing that surprised me is that we're essentially using all our own gear and medicine here at the hospital. We brought our own vest and compressor, and all our own medicines and vitamins. Good thing, too, especially for the enzymes, otherwise we'd be asking Lemon to wait 45 min before each meal or snack while an individual enzyme capsule was carried in by passenger pigeon from Nebraska.

Some other minor surprises include the fact that this room in a children's hospital doesn't have a changing table, which makes two kids in two different size diapers a real challenge, never mind the fact that they want us to save Lemon's diapers so they can measure his output. Also, room service will not deliver coffee, and you can't even call until 6:30 for your non coffee to be delivered, which doesn't help much if the kids are up at 5:15. Rest assured my little coffee pot from home is now installed in a spot befitting its importance in terms of my survival.

None of this would be as big a deal as it is were it not for the fact that we're not allowed to leave the room, lest we spread whatever Lemon has. I'm told there's a nice kitchenette on the floor with a refrigerator and hot water and whatnot, but it's of no more use to me than my kitchen at home. There's also a nice activity room and family lounge that we saw on our way in and won't see again until we leave.

So, that's our story. Lemon, Lime, and I in a hospital room together for the next 10-14 days, with our key support people and the ever changing roster of staff drifting in and out. We live so close to the hospital that I can see the foot of our street from our 5th floor window, and yet home sure seems pretty far away right now.

* written on my phone, so no photos this week--hopefully next week I'll figure out a way to get the pictures up.

Monday, December 7, 2015

As I mentioned last week, we introduced nebulized hypertonic saline to Lemon's treatment regimen, in the hopes that it will help him kick whatever it is that we've been treating with antibiotics for the last month. When we got our training last week, the respiratory therapist said that each treatment should take about 20 minutes. Well, we ran it at home and it took over 30 minutes. We've had this issue before, with our nebulized treatments taking much longer than they should. In fact, over the summer I was convinced that there was something wrong with the compressor that drives the nebulizer, and I exchanged our unit for a new one under warranty. That didn't make much difference, and we just sort of came to accept that the treatment we were doing took longer than it was supposed to.

That was all well and good when it was just one small (2.5mL) treatment, once a day. But, adding two additional treatments at 4mL each and we could see that all this nebulizing was going to dramatically increase our daily treatment time. And we don't have more time in the day, as previously discussed. I sent a message to our clinic explaining that our treatments were still taking too long, and asking for their suggestions. The message I got back said that the respiratory therapist forgot that we were doing our treatments at home (where did she imagine we were doing them, I wonder?), and had quoted us the time it would take with the equipment that they have at the hospital. Their proposed solution was to just run the nebulizer for 20 minutes each time, knowing that the full treatment wouldn't be delivered. But, they reasoned, Lemon is small and probably 2/3 of the dose would be enough.

I didn't find this answer very satisfying. First of all, Lemon won't always be small, and he will be doing nebulized treatments for the rest of his life. So we need a solution that will work both now and when he's bigger. Second, why not give him the full dose of each medication? If we're going to go to all the trouble of doing the treatments, I feel like we should do them right. Finally, the number of nebulized treatments that he has to do will only go up, so we need to solve this problem now, not later. It seemed like our clinic didn't have the answer I was looking for, so I took to the internet, and consulted some sources of unknown veracity, like, you know, blogs. And, I discovered that there is a whole category of compressors out there that for some reason our clinic never told us about, ones that can generate similar amounts of pressure to what they showed us at the hospital. Yes, they may not be covered by insurance. But we found one for $270 and thought it was worth a try, so we ordered it. Happy Hannukah, Lemon!

Here's a demonstration of the difference between the compressor the clinic gave us and the one we bought. In the video, the nebulizer connected to the new compressor (Mobileaire) is on the left, and the old (Innospire) on the right. My assistant's arm can be seen in the lower right. Look at the difference in output (mist) coming out of each one. Night and day, right? Now, we can get all our treatments done in about half the time as with the old compressor.

While I'm so glad that we were able to track down this solution, and are fortunate enough to have the resources to just buy it out of pocket, it's raised some questions for me. Why is it that our clinic never even mentioned this category of compressor to us as a possibility? Why did I have to use a combination of Google and looking at some of my favorite CF blogs to track this down? Why will insurance companies cover a nebulized medication that costs $1000+ per month, but balk at $270 for the machine that will allow you to actually deliver the treatment in a time short enough that it's compatible with the patient living a vaguely normal life?

Anyhow, enough on compressed air. Lemon came off antibiotics yesterday, so we're just keeping our fingers crossed that he stays healthy for the next few days/weeks and that we can avoid a hospitalization. Not that there's ever a good time for a hospitalization, but with Grandpa Dudley and Grandma Carol coming to visit for Christmas, and our much anticipated trip back to the east coast the following week, this would be a particularly bad time. Of course, we were not so foolish as to purchase any non-refunable tickets or hotels, but here's hoping that just for once we'll be able to execute a trip as planned!

Monday, November 30, 2015

Where are we? I'd say about 80% of the way there, which is not quite far enough. We started Lemon back on cefdinir (an antibiotic) a week ago, and it kicked in quickly. It got his cough back down from many, many times an hour to just a few times a day. We've done three sessions of the vest every day since last Sunday. We've added back two doses a day of Zyrtec to try to help dry up his various secretions. But, we are most definitely not at baseline. That wet cough is still lurking in the background, just waiting for a session of the vest or a dry bite of food to bring it to out. Lemon is feeling and acting fine, and yet things are not quite fine, and we are nervous about what will happen after this coming Sunday, when our second two-week cycle of cefdinir ends.

I do not like the feeling of being a sitting duck, so I called the clinic this morning and we managed to get in for an extra visit today before our extra visit next week. No surprise, Lemon has not gained any weight since last time, but he hasn't lost any either. He'd seemed so well in the waiting room that I was starting to question whether it was really worth bringing him in today, but he was eating snack when the nurse practitioner was in the room with us so she heard him cough several times and agreed that we are definitely not at baseline and need to get there whether we do it outpatient or in the hospital. She outlined three possible scenarios. One was to admit him today, one was to see if he manages to return to baseline by next Tuesday and if so just go on our merry way, and the third was to admit him from clinic next Tuesday if he isn't at baseline. We agreed that it seemed premature to admit him today, so our focus turned to how to increase the chances of option B over option C.

The one thing we had left to try was hypertonic saline, an inhaled solution of salt water that acts as an irritant to increase coughing and mucous clearance, and also helps to add moisture to the thick sticky mucous in CF lungs, allowing it to be cleared more easily. The nurse practitioner and our regular doctor had been hesitant to add it at this visit, because they weren't sure if we would be able to distinguish the increased cough that might result from adding hypertonic saline to our regimen from the increased cough that we're hearing as a result of this illness. I said I'd rather take that chance and try it than get admitted to the hospital next week knowing that there was one more thing we could have tried at home. If there's one thing I have expertise in at this point in my career as a CF mama, it is distinguishing between the many different types of coughs that a two year old can produce, so I think we'll be able to tell what's going on a week from now.

Of course, this decision extended our "brief" sick visit by an hour, since we had to try the first hypertonic saline treatment their in the clinic with the respiratory therapist, to make sure that Lemon didn't have any adverse reactions. The first step of the treatment is an albuterol inhaler, which relaxes the airways and makes it easier for the treatment to penetrate. The next step is the hypertonic saline itself, which takes about 20 minutes to nebulize. Luckily when we're at home we can do this at the same time as the vest, so it won't actually add more time to our treatment routine--just two more medications to manage and two more nebulizer cups per day to clean.

Lemon tolerated the treatment just fine, and as a reward for being an outstanding patient he received a new green truck from the clinic's prize bag. Of course, he was so thrilled by the prize that he couldn't eat his dinner, but that's a separate issue.

We'll begin integrating the new treatment into our routine tomorrow afternoon, once the medications are ready at our pharmacy. That will give us one chance to run through it with two adults in the house, before Papa Bear leaves for a quick trip to New York on Wednesday to see his side of the family, whom we haven't seen in entirely too long.

What else? Ah, there is Lime. Wonderful, chubby, cheerful, patient Lime. Full of smiles and laughter, figuring out his hands, resistant to sleeping anywhere that Mama is not, resistant to tasting cereal when nursing might be a possibility. Fascinated by his older brother, by the cats, and by anything with tiny plastic beads inside that make a little rattling noise. On the one hand I feel like I spend so many of my waking hours (of which there are very many) with him, and on the other hand I feel like I have so little time to just focus on him and drink in the joy of his babyhood with everything else that is going on. I decided a while ago that I would like him to have his very own episode of the blog, where he gets to take center stage at least for one week. Unfortunately, it can't be this week, and it probably can't be next week either. I hope for his sake and Lemon's that a quiet week comes quickly and we can all share in the bliss of a communal dose of babyhood, without the shadow of CF being quite as ominous as it is today.

Monday, November 23, 2015

Our adopted home state looks a little different since last our last update.

In our house, we scarcely need the visual reminder since we have a persistent audio reminder in the form of Lemon's cough, which is back with a vengeance. His last dose of antibiotics was on Wednesday of last week. The runny nose started up again almost immediately, followed as it inevitably is by the cough--the worst one he's ever had. We went up to 3 sessions/day of the vest on Sunday, and this morning first thing I was on the phone with the clinic to come up with a plan. We're back on oral antibiotics for another two weeks and reintroducing Zyrtec, and if he doesn't stay well after that, we're looking at the possibility of an admission. We've stared down these odds before, but that was in March with spring on the horizon, not in November which only inevitably leads to December. We're trying to remain optimistic, but it is decidedly harder at this time of year.

Aside from that, Mrs. Lincoln, it's been another normal-ish week at our house. Papa Bear left for a conference early Thursday morning, and Opa and Nona headed off to Chicago on Friday, so I was solo with the kids for a few days. It was actually fun in its own way, if you consider living in a sea of scattered toys and used tissues whilst going to bed at 8:30 and arising for the day several hours before dawn to be fun. We shoveled the driveway, went out for ice cream, went to the library, and did our weekly excursion to buy groceries and see fire trucks. The main thing is to completely and utterly surrender any hope that you (the adult) will actually accomplish a single thing for yourself. If you set your expectations to zero, you are much less likely to be disappointed. I managed a five minute phone call with an old friend and it felt like a major victory. We were all very glad when Papa Bear returned around mid-day Sunday. Lemon was especially delighted with the little souvenir that Papa had picked up for him in Orlando.
Looking ahead to this week, I am certainly glad that we set expectations to zero and made no plans whatsoever for Thanksgiving, so at least we won't have to cancel them. We're hoping that Lemon's cough responds to the antibiotics quickly, otherwise I'll be calling the clinic again Wednesday morning to see if we can get in for an appointment before the holiday. I suppose if we have to be admitted I'd rather it be from the clinic on Wednesday than from the ER over the holiday weekend.

In the spirit of Thanksgiving, I have to say that I am so thankful to be fighting this battle now, in 2015. I can listen to this cough and worry and fret, but I am worrying and fretting about his suffering, about adding new treatments, and the unknowns of a possible hospitalization, not that I might be in imminent danger of losing my child. I am so thankful that we have a wonderful care team that I trust, and that their efforts are guided by the research that was made possible by everyone who contributed their time, effort and money before Lemon was born. I am thankful to have the family that I have, and the support of all our friends near and far. I hope all of you have a wonderful Thanksgiving, and please eat an extra slice of pie for Lemon--he could use the calories!

Monday, November 16, 2015

This was our week of many appointments. First, Lemon had his clinic visit on Tuesday. To no one's surprise, he had gained barely 100g since his last visit, for a rate of 3g/day when our target is 5-10g/day. Of course this isn't ideal, but his doctor didn't seem overly alarmed. His feeling was that at least Lemon hadn't lost any weight, between his "experimental phases" and being sick last week. At least the antibiotics had worked quickly, and his cough was basically gone.

We are trying a few things to help with weight gain. First, we increased the dose of ranitidine (an acid reducing medication) that Lemon is on. He started on that to help raise his intestinal pH so that his enzymes could work better, and we haven't gone up on the dose in over a year. So, maybe we can gain a little ground just by re-optimizing his digestion. At his next appointment, he's due for an annual chest X-ray, so we're adding on an abdominal X-ray as well, to see if there is any stool built up in his intestines. Constipation is a very common problem in CF, and even "regular" CF patients sometimes have some build-up. If we see that on the X-ray, then we'll start him on Mirilax to try and clear it out, which will hopefully increase his appetite by making him feel less full. As you may imagine, the idea of giving a laxative to a two -year-old fills me with joyful anticipation, but whatever it takes, right? Finally, we're scheduling an appointment with a specialist at the clinic who may be able to give us some new strategies to encourage Lemon to eat a bit more. I still hold out hope that since what we have is essentially a behavioral problem, there will be a behavioral solution, and that solution might be more immediate than "wait until he's old enough that the behavior changes."

On Wednesday I took Lime to his 4 month checkup. He is officially a little teapot, short and stout, being in the 2nd percentile for height but the 20th for weight. He's growing right on his curve and has all the skills a 4 month old should have (including his first front-to-back rolls). So, we got a few shots and went home. Done. See you in 2 months. I'm really not used to this!

On Friday, around mid-morning, my phone rang and it was the CF clinic calling. I thought that was a bit odd, since they don't usually call except automated appointment reminders, and we didn't have any upcoming appointments. When I answered the phone, it was one of the nurses and I could tell by the tone of her voice that the message wasn't positive. My mind immediately leaped to the throat culture that was taken on Tuesday. Had they found something that they didn't want to find? The nurse asked me how Lemon was feeling, and whether he seemed lethargic. I said he was doing fine and was pretty much the opposite of lethargic. She said that was good, because there'd been a mistake and Lemon had been prescribed twice the dose of the antibiotic that he should have received. Since he was feeling well, the basic message was "no harm, no foul, go down to one dose a day instead of the two we told you to do and everything will be OK." Deeply reassuring.
Also deeply reassuring was the fact that this mistake was caught, not by the clinic, but by our insurance company who declined to pay for the prescription since the dose was out of range. The pharmacy was not thrilled about not being paid, and they called the clinic. It's sad that this mistake was only caught when there was money on the line, and sad that in spite of an electronic health record with a specific intervention plan detailed in it, that the doctor on call could have made this mistake. I am quite sure that the electronic health record system used by our hospital popped up an alarm when this prescription was entered, and that the doctor clicked away the alarm without really looking at it because there are a million alarms a day, most of them not meaningful, that he has to get through in order to actually do his job. So, added to the infinitely long list of tasks for the parents of a kid with a chronic illness is double-checking the dose on prescriptions, especially those done by someone other than our regular doctor.

Just to give Friday the 13th a little added kick, we got the news that Lemon's doctor will be leaving Madison come spring. This is pretty devastating news for us. We've seen him at the CF clinic every 6-8 weeks since we moved here, and I have so much respect for his knowledge and approach. We will probably switch to his senior colleague at the clinic, who is also a great doctor, so we will be in good hands. Still, I wasn't really prepared for this change and am feeling some trepidation about it. We cling to whatever is familiar and constant, especially when trying to navigate something as difficult CF, and Lemon's doctor has been a constant presence since we moved here. Mainly I wonder how we will find the bandwidth to navigate one more new thing, but I suppose we will find it somewhere.
At least I did get one reassuring piece of news on Friday--I got my teeth cleaned and everything was fine. Follow up in 6 months.

Monday, November 9, 2015

As of a week ago, Lemon had developed a bit of a cough. He was mostly coughing at night, although sometimes during the day as well. We upped his vest treatments to 3 times per day to see if that would shake the cough out of him, as it has for the previous two coughs he's had this fall. This one seems to have been made of sterner stuff, and he was still coughing on Friday. He seemed more or less his usual chipper self, but the cough was there and it sounded "juicy," as our nutritionist at the CF clinic describes these things. So, after some hemming and hawing, I called the clinic to request antibiotics. I wasn't eager to do it, since antibiotics come with their own problems and every use could increase the chance of developing resistance. But, with a cough lingering for 5 days, it was time.

We started the antibiotics on Friday evening, and as of today the cough is mostly gone. So, now after a full week of 90 min/day in the vest plus 3.5 days of antibiotics, we seem to be back on top in terms of pulmonary stuff. Of course, we still have 10 days of antibiotics to go and they have taken their usual toll on Lemon's delicate digestive system, resulting in really yucky poop and an incredibly painful diaper rash. It's also thrown off our usual system of giving all of Lemon's vitamins and supplements, because the antibiotic which has to be given twice a day with food can't be given in the same meal as his multivitamin, or his iron, or his probiotic, which we're supposed to increase to twice a day when he's on antibiotics. While it may sound like not a big deal (and in some sense it isn't), it means that nothing is happening at the time of day when it's supposed to, which just increases the chances that we will miss some doses of various things. It's not the end of the world, but it's not exactly ideal, either.

While all this was humming along in the background, I attended our CF center's annual Family Day on Saturday (video available here). I brought Lime with me, and he was definitely the star of the show. I do not exaggerate when I say that I had a line of people next to me offering to hold him for me. He was remarkably well behaved and took a long nap during the keynote presentation by Dr. Michael Boyle, Senior Vice President of Therapeutics Development at the Cystic Fibrosis Foundation. There's a lot of great stuff coming through the pipeline right now, from Vertex as well as other companies, and I continue to be very hopeful that before Lemon hits his teens there will be one or more good drugs available to help him.

Beyond getting an update on the state of new therapies, the real highlight of the day was getting a chance to catch up with other CF parents. As all parents know, having young children can be very isolating. Add to that having a young child with a rare disease, and add to that having a young child with a rare disease who specifically can never be in the same room as other people with the same disease, and you have a recipe for feeling like you're alone in the world. The instant camaraderie, even with CF parents that I'd never met before, was a huge source of comfort, and we're all hoping that we'll be able to set up some sort of recurring gathering more frequently than once per year.

This week and next are likely to be rodeos--tomorrow is Lemon's clinic visit, Wednesday is Lime's 4 month (!!!) check up, and Friday I'm going to the dentist. All during business hours of course, so who knows exactly when I'll actually do my job. And
then at the end of next week, I'll be flying solo as Papa Bear heads to a
conference and Opa and Nona take some well-deserved R&R in the
windy city. And the week after that is Thanksgiving. Let's do this,
November!